TY - JOUR
T1 - EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis
T2 - Ankara 2008. Part I: Overall methodology and clinical characterisation
AU - Ruperto, Nicolino
AU - Ozen, Seza
AU - Pistorio, Angela
AU - Dolezalova, Pavla
AU - Brogan, Paul
AU - Cabral, David A.
AU - Cuttica, Ruben
AU - Khubchandani, Raju
AU - Lovell, Daniel J.
AU - O'Neil, Kathleen M.
AU - Quartier, Pierre
AU - Ravelli, Angelo
AU - Iusan, Silvia M.
AU - Filocamo, Giovanni
AU - Magalhães, Claudia Saad
AU - Unsal, Erbil
AU - Oliveira, Sheila
AU - Bracaglia, Claudia
AU - Bagga, Arvind
AU - Stanevicha, Valda
AU - Manzoni, Silvia Magni
AU - Pratsidou, Polyxeni
AU - Lepore, Loredana
AU - Espada, Graciela
AU - Paut, Isabelle Kone
AU - Zulian, Francesco
AU - Barone, Patrizia
AU - Bircan, Zelal
AU - Maldonado, Maria Del Rocio
AU - Russo, Ricardo
AU - Vilca, Iris
AU - Tullus, Kjell
AU - Cimaz, Rolando
AU - Horneff, Gerd
AU - Anton, Jordi
AU - Garay, Stella
AU - Nielsen, Susan
AU - Barbano, Giancarlo
AU - Martini, Alberto
PY - 2010/5
Y1 - 2010/5
N2 - Objectives: To report methodology and overall clinical, laboratory and radiographic characteristics for Henoch-Schönlein purpura (HSP), childhood polyarteritis nodosa (c-PAN), c-Wegener granulomatosis (c-WG) and c-Takayasu arteritis (c-TA) classification criteria. Methods: The preliminary Vienna 2005 consensus conference, which proposed preliminary criteria for paediatric vasculitides, was followed by a EULAR/PRINTO/PRES - supported validation project divided into three main steps. Step 1: retrospective/prospective web-data collection for HSP, c-PAN, c-WG and c-TA, with age at diagnosis ≤18 years. Step 2: blinded classification by consensus panel of a subgroup of 280 cases (128 difficult cases, 152 randomly selected) enabling expert diagnostic verification. Step 3: Ankara 2008 Consensus Conference and statistical evaluation (sensitivity, specificity, area under the curve, κ-agreement) using as 'gold standard' the final consensus classification or original treating physician diagnosis. Results: A total of 1183/1398 (85%) samples collected were available for analysis: 827 HSP, 150 c-PAN, 60 c-WG, 87 c-TA and 59 c-other. Prevalence, signs/symptoms, laboratory, biopsy and imaging reports were consistent with the clinical picture of the four c-vasculitides. A representative subgroup of 280 patients was blinded to the treating physician diagnosis and classified by a consensus panel, with a κ-agreement of 0.96 for HSP (95% CI 0.84 to 1), 0.88 for c-WG (95% CI 0.76 to 0.99), 0.84 for c-TA (95% CI 0.73 to 0.96) and 0.73 for c-PAN (95% CI 0.62 to 0.84), with an overall κ of 0.79 (95% CI 0.73 to 0.84). Conclusion: EULAR/PRINTO/PRES propose validated classification criteria for HSP, c-PAN, c-WG and c-TA, with substantial/almost perfect agreement with the final consensus classification or original treating physician diagnosis.
AB - Objectives: To report methodology and overall clinical, laboratory and radiographic characteristics for Henoch-Schönlein purpura (HSP), childhood polyarteritis nodosa (c-PAN), c-Wegener granulomatosis (c-WG) and c-Takayasu arteritis (c-TA) classification criteria. Methods: The preliminary Vienna 2005 consensus conference, which proposed preliminary criteria for paediatric vasculitides, was followed by a EULAR/PRINTO/PRES - supported validation project divided into three main steps. Step 1: retrospective/prospective web-data collection for HSP, c-PAN, c-WG and c-TA, with age at diagnosis ≤18 years. Step 2: blinded classification by consensus panel of a subgroup of 280 cases (128 difficult cases, 152 randomly selected) enabling expert diagnostic verification. Step 3: Ankara 2008 Consensus Conference and statistical evaluation (sensitivity, specificity, area under the curve, κ-agreement) using as 'gold standard' the final consensus classification or original treating physician diagnosis. Results: A total of 1183/1398 (85%) samples collected were available for analysis: 827 HSP, 150 c-PAN, 60 c-WG, 87 c-TA and 59 c-other. Prevalence, signs/symptoms, laboratory, biopsy and imaging reports were consistent with the clinical picture of the four c-vasculitides. A representative subgroup of 280 patients was blinded to the treating physician diagnosis and classified by a consensus panel, with a κ-agreement of 0.96 for HSP (95% CI 0.84 to 1), 0.88 for c-WG (95% CI 0.76 to 0.99), 0.84 for c-TA (95% CI 0.73 to 0.96) and 0.73 for c-PAN (95% CI 0.62 to 0.84), with an overall κ of 0.79 (95% CI 0.73 to 0.84). Conclusion: EULAR/PRINTO/PRES propose validated classification criteria for HSP, c-PAN, c-WG and c-TA, with substantial/almost perfect agreement with the final consensus classification or original treating physician diagnosis.
UR - http://www.scopus.com/inward/record.url?scp=77951583607&partnerID=8YFLogxK
U2 - 10.1136/ard.2009.116624
DO - 10.1136/ard.2009.116624
M3 - Article
C2 - 20388738
AN - SCOPUS:77951583607
SN - 0003-4967
VL - 69
SP - 790
EP - 797
JO - Annals of the Rheumatic Diseases
JF - Annals of the Rheumatic Diseases
IS - 5
ER -